Scleroderma-like Fibrosing Disorders

Boin, Francesco; Hummers, Laura K.

doi:10.1016/j.rdc.2007.11.001

Cited by 111 publications

(90 citation statements)

References 97 publications

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“…However, it is a rare entity of unknown etiology. [5,6] Moreover, the patient was a smoker and the angiographyconfirmed peripheral vascular disease aggravated his leg ulcers.…”

Section: Discussionmentioning

confidence: 98%

Sometimes patience pays off: When amputation is the alternative

Delikonstantinou

Zapantioti

Gravvanis

et al. 2016

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“…However, it is a rare entity of unknown etiology. [5,6] Moreover, the patient was a smoker and the angiographyconfirmed peripheral vascular disease aggravated his leg ulcers.…”

Section: Discussionmentioning

confidence: 98%

Sometimes patience pays off: When amputation is the alternative

Delikonstantinou

Zapantioti

Gravvanis

et al. 2016

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“…Pode atingir a face, braços e restantes partes do tronco, mas as mãos e pés são tipicamente poupados 1,3,4 . As manifestações sistêmicas da doença são raras, podendo haver envolvimento muscular (miosite), ocular (paralisia músculos oculares), gastrointestinal (disfagia), respiratório e cardíaco 5 . Estão descritos resultados divergentes quanto à prevalência, variando consoante os estudos entre 2,5% até 14% 6,7,8 .…”

Section: Discussionunclassified

ESCLEREDEMA DE BUSCHKE – A propósito de um caso clínico

Henriques¹,

Almeida²,

Araújo³

et al. 2017

Gal. Clin.

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RESUMOO Escleredema de Buschke (EB) é uma doença rara do tecido conjuntivo, de etiologia desconhecida, caracterizada por espessamento e endurecimento da pele, geralmente localizado nas regiões proximais e posteriores do tronco e pescoço. Existem três formas clínicas com manifestações, evolução e prognóstico diferentes, classificadas de acordo com a patologia associada: Tipo 1 (forma clássica), surge após um episódio infecioso (particularmente por Streptoccocus) e tem resolução espontânea; o Tipo 2 está associado a paraproteinemias o Tipo 3 ou diabeticorum, surge de forma insidiosa em pessoas com Diabetes mellitus (DM) com uma longa história de mau controlo metabólico. A presença de epiderme normal com derme espessada e aumento de espaços entre grandes feixes de colagé-nio, resultante da deposição de mucopolissacarídeo (ácido hialurônico) na biópsia cutânea confirma o diagnóstico. Não há uma terapêutica padrão, pois o sucesso terapêutico na literatura é limitado a relatos de casos. Os autores relatam um caso de EB numa doente com DM tipo 2 de longa data com mau controlo metabólico e complicações microvasculares. Palavras chave: escleredema adultorum, escleredema diabeticorum, diabetes mellitus, fototerapia, PUVA. ABSTRACTScleredema of Buschke (EB) is a rare sclerotic disorder, of unknown etiology, characterized by a thickening of the reticular dermis usually localized in the upper back of the trunk and neck. There are three clinical forms, which are classified by their associated condition: type 1, the classical form, suddenly appears after infectious episode (particularly streptococcal infection) and have spontaneous resolution; type 2 is associated with paraproteinemias; and type 3, diabeticorum, appears more insidiously associated with diabetes mellitus with a long history of poor metabolic control. The histologic finding of a normal epidermis with a thickened dermis and increased spaces between large collagen bundles, resulting from deposition of mucopolysaccharide (hyaluronic acid) in the dermis in skin biopsy confirm the diagnosis. There is no standard therapeutic protocol, as therapeutic success in the literature is limited to case reports. The authors report a case of EB in patients with long-standing type 2 diabetes mellitus, poor metabolic control and microvascular complications.

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“…In these patients, the use of other immunosuppressive agents, including methotrexate, hydroxychloroquine and others, might be necessary. The prognosis is usually favourable, as complete remission is generally achieved, even if prolonged or combined immunosuppressive treatment is necessary [7]. The absence of clinical response should prompt further investigation to rule out an underlying malignancy.…”

Section: Discussionmentioning

confidence: 99%